Download PowerPoint Presentation - Neuro-Ophthalmology and Multiple

The Neuro-Ophthalmology
of Multiple Sclerosis
Charles Maxner MD, FRCPC
Professor, Departments of Medicine (Neurology) and
Ophthalmology
Dalhousie University
Consultant, Dalhousie MS Research Unit
Halifax , N.S.
Dr. C.E. Maxner: Disclosure
Dr. Maxner has attended and conducted
educational events and participated in MS
research studies affiliated with the following
firms:
Berlex
Biogen Idec
Serono
Teva
The Visual System
and MS
Objectives:
Briefly review MS as a disorder
Review how it affects:
The Afferent Visual System
The Efferent Visual System
MS: Historical Perspective
Augustus d’Esté (1794-1848)
J.M. Charcot (1825-1893)
Grandson King George III
(1868 leçons: ”sclérose en
plaques disseminées” from
Vulpian)
Carswell ~1836
Multiple Sclerosis
Disorder of Central Myelin (Oligodendroglia)
Brain and Spinal Cord
Immune Based
Inflammatory demyelinating disorder
Axonal injury (Disability)
Multiple Sclerosis:
3 Components
Inflammation
Demyelination
Axonal Loss
Courtesy Dr. G. Rice
Multiple Sclerosis
Pathology
Gross Pathology
Luxol Fast Blue
Multiple Sclerosis
Pathology
Optic Nerves
Chiasm
Optic Tract
Anterior Visual Pathway
Luxol Fast Blue
Action Potential Transit in MS
Concepts
1. Delayed Conduction
2. Conduction Block
Courtesy Dr. A.Bar-Or
Natural Progression of MS
Relapsing Forms
Relapsing-Remitting
Clinically
definite MS
Secondary Progressive
Relapse
Clinical Worsening
MonoSubclinical symptomatic
Initial
demyelinating
event
Time
Level of disability
Gadolinium enhancement
Cognitive dysfunction
Relapses
Accumulated MRI lesion burden
Brain atrophy
Courtesy Dr. G. Rice
MRI Dissemination in
Space and Time
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Presenting Symptoms of MS
Symptom
Weakness in one or more
limbs
Sensory loss/paresthesias
Visual loss
Gait disturbance/ataxia
Diplopia
Dizziness/vertigo
Pain
Sensory in face
Approximate Prevalence
40-50%
40-45%
16-36%
5-15%
7-15%
5%
3%
3%
Neuro-ophthalmological
Issues
Loss of Vision
(Monocular and Binocular)
Diplopia
Oscillopsia
MS and the Visual System
Afferent Visual System
Vision loss and distortion
Efferent Visual System
Diplopia and Oscillopsia
MS and The Afferent
Visual System
Pre-chiasmal
Optic Nerve
Chiasmal
Bitemporal VF defect rare
Junctional Scotoma defect not uncommon
Post-Chiasmal
Optic tract
Geniculocalcarine pathway
Case: Ms. H.B. 35 YOWF
MS diagnosed 12 years prior
Copaxone Therapy
Decreased vision left eye
Progressed over 48 hours
Pain on eye movement
Impaired depth perception
“Can’t drive”
Case: Ms. H.B. 35 YOWF
Examination
Va 6/6 Right, HM Left
Central scotoma left eye
RAPD 1.5 log units left eye
Impaired colour perception left
Ocular motility normal
Left disc slightly swollen and hyperemic
Pupil Testing
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Case: Ms. H.B. 35 YOWF
Goldmann Visual Fields
Case: Ms. H.B. 35 YOWF
Follow Up: 3 months later
Va 6/6 Right, 6/9 Left
Central blur left eye
RAPD 0.6 left eye
Colour improved
Temporal pallor left disc
Optic Neuritis
Common Symptoms
Monocular
Central Vision loss
Pain (eye movement)
Altered colour vision
Recovery common
Uhthoff’s symptom
Flashes
Pulfrich phenomenon
Uhthoff’s Symptom
What did he describe?
Uhthoff described 3 patients in whom exertion
and fatigue caused a desaturation in colour
vision
Patient XVIII had decreased acuity after
walking around the room
Who was Uhthoff?
Uhthoff’s Symptom
Wilhelm Uhthoff (1853-1927)
Born Warin , Germany
Studied in Tübingen, Göttingen, Berlin
Consultant at Westphal’s Clinic (With Oppenheim,
Wallenberg, Thomsen, Möbius)
Named Professor of Ophthalmology at Breslau 1896
Eye Symptoms in Diseases of the Nervous System
(Published 1915)
Described by Bielschowsky as the “true originator”
of clinical neuro-ophthalmology
Wilhelm Uhthoff
Uhthoff’s Symptom
Uhthoff’s symptom in optic
neuritis:relationship to MRI and
development of MS. (Scholl GB, Song
HS, Wray SH) Ann Neurol 1991; 30(2):1804
Uhthoff and his Symptom (Selhorst JB,
Saul RF) Journal of Neuro-ophthalmology
1995; 15(2):63-9
Flashes
Movement phosphenes in optic
neuritis: A new clinical sign (Davis F,
Bergen D, Schauf C, McDonald I, Deutsch W)
Neurology 1976; 26: 1100-1104.
Bright flashes in dark
Eye movement
Differentiate from Lightning Streaks of Moore
Eye equivalent of Lhermittes symptom
Pulfrich Phenomenon
Optic Neuritis: Physical
Findings
Decreased visual acuity
VF defect
(Central/Altitudinal 29% )
Dyschromatopsia
Afferent Pupil Defect
(RAPD)
Optic disc swelling 35%
Abnormal Contrast
Sensitivity
Abnormal VEP
Altered Flicker
Perception
Altered depth perception
Optic disc pallor
Optic Neuritis: Optic Disc
Case: Ms. A.B. 23 YOWF
Two months impaired vision both eyes
Progressive course
Blurred centrally right eye
Hazy to left of fixation both eyes
Occasional migraine
Case: Ms. A.B. 23 YOWF
Va 6/15 Right, 6/7.5 Left
Confrontation VF: Left Central HH
No RAPD
AO Plates: 7/14 Rt 10/14 Lt
Ocular motility normal
Anomalous discs both eyes
Case: Ms. A.B. 23 YOWF
Automated Perimetry
Case: Ms. A.B. 23 YOWF
MRI Imaging
Case: Ms. C.S. 41 YOWF
2 week hx of L sided visual blurring
Both eyes involved
15 years ago “poor balance”
Migraines
Sister with MS
Case: Ms. C.S.
Examination
Va: 6/6 Both Eyes
AO Plates: 13/14 Rt, 11/14 Lt
Pupils normal
Ocular motility normal
Fundi normal
DTR’s brisk, Unsteady Romberg
VF’s abnormal
Ms. C.S. Visual Fields
Ms. C.S. MRI
Ms. C.S. MRI (2 mos later)
Optic Neuritis:
The Differential
AION (Ischemic Optic Neuropathy)
 Vasculitic Disorders (i.e. SLE)
 Hereditary (i.e. Leber’s)
 Toxic/Nutritional (ETOH)
 Infectious (i.e.Bartonella, Lyme)
 Inflammatory (i.e. Sarcoid)
 Neoplastic/Paraneoplastic (i.e. lymphoma)
 Compressive (i.e.Tumours, Grave’s orbitopathy)
 Amblyopia

Neuro-ophthalmological
Issues
Diplopia
Horizontal, Vertical, Mixed
Fluctuating
Oscillopsia
Ocular Motility Disorders
Infranuclear or Nerve
Saccadic system
Pursuit system
Internuclear abnormalities
Vestibulo-ocular dysfunction
Nystagmus
Ocular Motility Disorders
Nuclear Palsies: Rare
Infranuclear or Nerve
VI: Most common
III: Partial or Complete
IV: Rare
Ms. H.M. 34 YOWF
CC: Diplopia
Hx: 6 months progressing diplopia
Initially intermittent, now persistent
Otherwise asymptomatic
Sister has MS
O/E: Incomitant esotropia
Left abduction deficit
Ms. H.M. 34 YOWF
Ms. H.M. 34 YOWF
6 Months Later
Ocular Motility Disorders
Saccadic abnormalities
Hypometric
Hypermetric
Dysmetria
Saccadic Intrusions
Square wave jerks
Saccadic pulses
Ocular flutter
Saccadic Abnormalities
From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company
Saccadic Oscillations
Saccadic Dysmetria
Macrosaccadic Oscillations
Square Wave Jerks
Macro Square Wave Jerks
Ocular Flutter
From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company
Ocular Motility Disorders
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Square Wave Jerks
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Ocular Flutter
Ocular Motility Disorders
Pursuit Dysfunction
Saccadic Intrusions
Internuclear Ophthalmoplegia
MLF Lesion
Skew Deviation
Vertical diplopia
Gaze Palsies
Dorsal Midbrain Syndrome
Ocular Motility Disorders
Pursuit Dysfunction
Saccadic Intrusions
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Ocular Motility Disorders
Internuclear Ophthalmoplegia:MLF Lesion
From: Kline & Bajandas. Neuro-ophthalmology Board Review Manual; Slack Inc
Ms.C.P. 24 YOWF
CC: Blurred Vision
Hx:
• 2 week history of “dizzy” feeling and
disorientation with looking down
• Difficulty focussing on rapid EOM’s
• 2003 sensory symptoms in legs and Lhermittes
symptom
O/E: Abnormal EOM’s
Ms.C.P. 24 YOWF
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Ms.C.P. 24 YOWF
T2
Flair
Internuclear Ophthalmoplegia
MRI Detection of MLF Lesions
Proton density>T2>Flair
Frohman et al Neurology 2001; 57:762-768
Proton
Density
T2
Flair
Internuclear Ophthalmoplegia
Versional Disconjugacy Index: Assess adduction vs
abduction saccade peak velocity
Most accurate method for identification of INO is
quantitative EOM recording
Clinical detection accuracy vs Recording
93% severe INO
75% moderate INO
29% mild INO
Frohman et al. Neurology 2003;61:848-850
Ocular Motility Disorders
Vestibulo-ocular Dysfunction
VOR Mismatch
Failure of VOR Suppression
Vestibulo-Ocular reflex
From: Leigh & Zee. The Neurology of Eye Movements, F.A. Davis Company
Vestibulo-Ocular reflex
From: Kline & Bajandas. Neuro-ophthalmology Board Review Manual; Slack Inc
Head Thrust Test
Halmagyi Maneuver
Thrust head 20-30 degrees while fixating target
Abnormal:
Refixation saccade
Headshake Test
Shake head for 20 seconds at 2 hz (horizontal
and vertical) with eyes closed, then open
and observe for nystagmus (Frenzel lenses)
Abnormal:
Unidirectional nystagmus in plane of
headshake (peripheral)
Vertical nystagmus after horizontal
headshake (central)
Dynamic Visual Acuity Test*
Read eye chart with eyes open and with slow
head shake
Abnormal:
>3 line drop in acuity
* VOR test
Fixation Suppression Test*
Fixate own thumb while chair rotates
Abnormal:
Nystagmus in direction of rotation
* VOR suppression test
Failure of VOR Suppression
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Ophthalmoscopic Testing
Spontaneous nystagmus
Retinal slip: Observe fundus while patient fixates
target and oscillates head at frequency greater than
1 cps
Abnormal: If the VOR gain is too high the disc
appears to move with the head , if too low,
opposite the head
Provocative Testing
Caloric stimuli
Hyperventilation
Pressure stimulus
Sound stimulus (Tullio’s Phenomenon )
Nystagmus
Interesting but rarely localizing
Gaze evoked
Direction changing
cerebellar
Direction selective
vestibular
Ataxic of INO
Vertical (Upbeat or
downbeat)
Rebound
Torsional
Acquired pendular
Periodic alternating
Lid nystagmus
Superior oblique
myokymia*
*Not really a “nystagmus”
Nystagmus(es) in MS Patient
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Ocular Motility Disorders
There are some ocular motility disturbances that have nothing to do with MS.
Congenital strabismus
Latent nystagmus
DVD (Dissociated Vertical Divergence)
Convergence spasm
Voluntary nystagmus
Congenital or chronic IVth (FAT scan)
Duane’s Retraction Syndrome
What is this?
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Jelly nystagmus: Constant Ocular Oscillation seen
in association with poor vision
Thank You !
Time for
Questions